Primary Hemophagocytic Lymphohistiocytosis: A Severe Immune Dysregulatory Disease with Various Genotypic Features and Outcomes: A Cross-Sectional Study from a Tertiary Pediatric Center

doi:10.4274/tjh.galenos.2025.2025.0221

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Article

Primary Hemophagocytic Lymphohistiocytosis: A Severe Immune Dysregulatory Disease with Various Genotypic Features and Outcomes: A Cross-Sectional Study from a Tertiary Pediatric Center

Turkish journal of haematology : official journal of Turkish Society of Haematology - 20 Feb 2026

Öztürk Ahmet Gökcan, Haskoloğlu Zehra Şule, Çakmaklı Hasan Fatih, İslamoğlu Candan, İleri Talia, İnce Elif, İncesoy Özdemir Sonay, Dinçaslan Handan Uğur, Taçyıldız Nurdan, Cabı Ünal Emel, Doğu Figen, Ertem Mehmet, İkincioğulları Aydan

Abstract excerpt

Objective: Inborn errors of immunity (IEIs) are caused by deficiencies or functional abnormalities in the immune system, leading to increased susceptibility to infections, autoimmunity, autoinflammatory diseases, allergies, and/or malignancies. Primary hemophagocytic lymphohistiocytosis (HLH) arises from genetic mutations affecting the function of cytotoxic T lymphocytes and natural killer cells, while secondary...

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PMID: 41437329
DOI: 10.4274/tjh.galenos.2025.2025.0221
PMCID: PMC12922558

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